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Unique lymphocyte subsets, including activated na\u00efve and transitional B cells, CD19\n                      <jats:sup>hi<\/jats:sup>\n                      CD21\n                      <jats:sup>lo<\/jats:sup>\n                      B cells, follicular helper T (T\n                      <jats:sub>FH<\/jats:sub>\n                      ) cells, regulatory T (T\n                      <jats:sub>reg<\/jats:sub>\n                      ) cells, and TCR\u03b1\u03b2\n                      <jats:sup>+<\/jats:sup>\n                      CD4\n                      <jats:sup>\u2212<\/jats:sup>\n                      CD8\n                      <jats:sup>\u2212<\/jats:sup>\n                      double\u2010negative T cells (DNT\u03b1\u03b2), were assessed by flow cytometry. Serum levels of lipopolysaccharide (LPS), B\u2010cell activating factor (BAFF), and soluble IL\u20102 receptor (sIL2R) were quantified by ELISA.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 104 AIC patients, 53 (51%) showed evidence of IEI, including 27 (26%) with monogenic disorders\u2014most commonly partial DiGeorge syndrome (pDGS), followed by variants in NFKB1, CTLA4, and FAS. The prevalence of IEI was highest in autoimmune hemolytic anemia (AIHA) (62.5%) and Evans syndrome (61.5%). Low levels of IgG, IgA, and IgM, as well as reduced percentages of na\u00efve CD4\n                      <jats:sup>+<\/jats:sup>\n                      and CD8\n                      <jats:sup>+<\/jats:sup>\n                      T cells, were significantly associated with increased odds of IEI. In AIC\u2010IEI patients, transitional B cells, CD19\n                      <jats:sup>hi<\/jats:sup>\n                      CD21\n                      <jats:sup>lo<\/jats:sup>\n                      B cells, and T\n                      <jats:sub>FH<\/jats:sub>\n                      cells were expanded, accompanied by elevated serum levels of BAFF and sIL2R.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Quantitative immunoglobulin levels and na\u00efve T cells remain valuable indicators of IEI in AIC. Our findings highlight the diagnostic value of emerging cellular and serum biomarkers in identifying IEI, including dysregulation of early B\u2010cell subsets (transitional B cells and CD19\n                      <jats:sup>hi<\/jats:sup>\n                      CD21\n                      <jats:sup>lo<\/jats:sup>\n                      B cells), expansion of T\n                      <jats:sub>FH<\/jats:sub>\n                      cells, and elevated levels of BAFF and sIL2R.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/1545-5017.70074","type":"journal-article","created":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T08:30:37Z","timestamp":1768984237000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Investigating Biomarkers for Inborn Errors of Immunity in a Prospective Study of Patients With Autoimmune 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